TY - JOUR
T1 - First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome
AU - Rigante, Donato
AU - Capoluongo, Ettore Domenico
AU - Bertoni, Barbara
AU - Ansuini, Valentina
AU - Chiaretti, Antonio
AU - Piastra, Marco
AU - Pulitano', Silvia Maria
AU - Genovese, Orazio
AU - Compagnone, Adele
AU - Stabile, Achille
PY - 2007
Y1 - 2007
N2 - We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up-regulated monokine production, high serum levels of triglycerides and ferritin, clotting abnormalities with hypofibrinogenemia, and rapidly evolving pancytopenia should alert the clinician to the possible diagnosis of macrophage activation syndrome, even in autoinflammatory diseases characterized basically by the periodic recurrence of unprovoked inflammatory attacks. Bone marrow aspiration showing well-differentiated macrophages phagocytosing hematopoietic elements remains the main tool for a final diagnosis, and cyclosporine is the best strategy for treatment.
AB - We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up-regulated monokine production, high serum levels of triglycerides and ferritin, clotting abnormalities with hypofibrinogenemia, and rapidly evolving pancytopenia should alert the clinician to the possible diagnosis of macrophage activation syndrome, even in autoinflammatory diseases characterized basically by the periodic recurrence of unprovoked inflammatory attacks. Bone marrow aspiration showing well-differentiated macrophages phagocytosing hematopoietic elements remains the main tool for a final diagnosis, and cyclosporine is the best strategy for treatment.
KW - hyperIgD-syndrome
KW - macrophage activation syndrome
KW - hyperIgD-syndrome
KW - macrophage activation syndrome
UR - https://publicatt.unicatt.it/handle/10807/3667
UR - https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=33847068680&origin=inward
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=33847068680&origin=inward
U2 - 10.1002/art.22409
DO - 10.1002/art.22409
M3 - Article
SN - 0004-3591
VL - 2007
SP - 658
EP - 661
JO - Arthritis and Rheumatism
JF - Arthritis and Rheumatism
IS - 56(2)
ER -